Division of Hematology with BMT, Department of Oncology, University of Palermo, Palermo, Italy.
Acta Haematol. 2010;124(1):5-8. doi: 10.1159/000314900. Epub 2010 May 26.
T cell large granular lymphocytic (LGL) leukemia is a rare condition accounting for 2-3% of all mature lymphoid leukemias. Here, we present the case of a 73-year-old woman presenting with neutropenia and anemia (hemoglobin 9.9 g/dl). Hematological assessment revealed the presence of a T cell LGL leukemia. At the time of T cell LGL leukemia diagnosis, the patient developed xerophthalmia and xerostomia, and a diagnosis of Sjögren's syndrome was made following salivary gland biopsy. The finding of large granular lymphocytes in the context of autoimmune disorders is well-known, though it often occurs with rheumatoid arthritis or in association with a positive autoantibody titer in the absence of an overt clinical picture. The concomitant presentation of T cell LGL leukemia with Sjögren's syndrome is a rare event which is worth reporting. Our patient was managed with immunosuppressive therapy and is still alive.
T 细胞大颗粒淋巴细胞（LGL）白血病是一种罕见的疾病，占所有成熟淋巴细胞白血病的 2-3%。在这里，我们报告了一例 73 岁女性因中性粒细胞减少和贫血（血红蛋白 9.9g/dl）就诊的病例。血液学评估显示存在 T 细胞 LGL 白血病。在诊断为 T 细胞 LGL 白血病时，患者出现干眼症和口干症，并在唾液腺活检后诊断为干燥综合征。自身免疫性疾病中出现大颗粒淋巴细胞是众所周知的，但它通常与类风湿关节炎有关，或者在没有明显临床表现的情况下与自身抗体滴度阳性相关。T 细胞 LGL 白血病与干燥综合征同时发生是一种罕见事件，值得报告。我们的患者接受了免疫抑制治疗，目前仍存活。
